The Cytology of Metastatic Angiosarcoma in Pleural Fluid - A Case Report -

김나래,정동해,조현이 대한병리학회 2009 Journal of Pathology and Translational Medicine Vol.43 No.3

A 74-year-old woman presented with an abrupt onset of dyspnea that she had experienced for a week. She had been suffering from cutaneous nodules in the scalp for a year. Thoracentesis of the pleural fluid was performed. The Papanicolaou-stained smears, Thin prep and cell block preparations revealed clusters of oval-shaped cells concentrically layered about amorphous acellular cores, i.e., there was microacinar lumen formation as well as singly scattered atypical cells. The cells occasionally demonstrated intracytoplasmic vacuoles and hemosiderin deposits. Those cells stained for CD31 and they were negative for pancytokeratin. Punch biopsy from the scalp nodules revealed angiosarcoma. There are currently few reported cases of angiosarcoma in an exfoliative pleural effusion. Angiosarcoma has diverse, heterogeneous cytologic features. Making the cytologic diagnosis of metastatic angiosarcoma in pleural fluid is a challenge for pathologists. Knowledge of the clinical history is of great help for diagnosing this tumor when it appears in rare sites. Immunopanels with CD31, pancytokeratin and TTF-1 are helpful for making the differential diagnosis. The pathologists should look for clues suggesting the presence of vascular differentiation in the exfoliative cytologic materials when a diagnosis of angiosarcoma is suspected. A 74-year-old woman presented with an abrupt onset of dyspnea that she had experienced for a week. She had been suffering from cutaneous nodules in the scalp for a year. Thoracentesis of the pleural fluid was performed. The Papanicolaou-stained smears, Thin prep and cell block preparations revealed clusters of oval-shaped cells concentrically layered about amorphous acellular cores, i.e., there was microacinar lumen formation as well as singly scattered atypical cells. The cells occasionally demonstrated intracytoplasmic vacuoles and hemosiderin deposits. Those cells stained for CD31 and they were negative for pancytokeratin. Punch biopsy from the scalp nodules revealed angiosarcoma. There are currently few reported cases of angiosarcoma in an exfoliative pleural effusion. Angiosarcoma has diverse, heterogeneous cytologic features. Making the cytologic diagnosis of metastatic angiosarcoma in pleural fluid is a challenge for pathologists. Knowledge of the clinical history is of great help for diagnosing this tumor when it appears in rare sites. Immunopanels with CD31, pancytokeratin and TTF-1 are helpful for making the differential diagnosis. The pathologists should look for clues suggesting the presence of vascular differentiation in the exfoliative cytologic materials when a diagnosis of angiosarcoma is suspected.

두피에 발생한 혈관육종의 치험 2례

양은진,김정태,김연환,이형중 대한성형외과학회 2009 Archives of Plastic Surgery Vol.36 No.1

Purpose: Angiosarcoma of the scalp is unusual vascular tumor originating from endothelial cell. Angiosarcoma is an aggressive tumor with high propensity for both local recurrence and distant metastasis. We report 2 cases of angiosarcoma having poor prognosis recently. Methods: Case 1 was a 67-year-old male patient. He visited the hospital with a 3×5cm sized discolored mass in forehead. It began at one month ago from coming to the hospital. Case 2 was a 64-year-old male patient. He visited for our hospital to remove a 4×5cm sized scalp mass. He had a pruritis on scalp from 9 months ago before coming to the hospital. Despite of the conservative treatments, the wound was not healed and advanced necrotic lesion with hemorrhage. Results: Case 1 was diagnosed as an angiosarcoma. He underwent a radical operation 5 times. But the tumor expanded multiple area in dura & frontal area of the brain. The patient rejected the treatment any more. And he died one month later. Case 2 was diagnosed as an angiosarcoma and metastased to skull in MRI. He got a radical resection including cranium. Three months later, it recurred to ipsilateral Sternocleidomastoideus muscle. He got a additional operation & started radiotherapy. And now he is receiving chemotherapy, but the recurred lesion is expanding. Conclusion: Angiosarcoma is a highly malignant tumor. Especially it arises in vascularized area, it easily metastases. So it is best to treat angiosarcoma with surgery included wide margins. Despite the small lesion, we remember that angiosarcoma is a tumor that was consider to be metastases.

[P204] A case of retiform hemangioendothelioma further diagnosed as angiosarcoma; is it a disease spectrum from retiform hemangioendothelioma to angiosarcoma?

( Yeongjoo Oh ),( Hemin Lee ),( Sang Ho Oh ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Hemangioendothelioma(HE) is considered to be a disease entity which has characteristics between completely benign hemangioma and malignant angiosarcoma. Retiform hemangioendothelioma (RH), also known as a “Hobnail HE”, has been reported as a distinct form of low grade angiosarcoma because it has a slow indolent course. A 56 years old female patient presented with diffuse erythematous to purpuric plaque on her scalp for 10 years. Skin pathology showed rete testis form anastomosing vessels, hobnail-like endothelial cells lining the vessels, consistent with the findings of RH. On whole body CT and PET-CT, done for systemic evaluation, enhancement of multiple neck lymph nodes was observed. And ultrasound-guided needle aspiration biopsy from neck node appeared typical pathologic findings of angiosarcoma differently from original mass of RH. RH is a rare vascular neoplasm with very high local recurrence and a very rare metastasis. However, this patient diagnosed as RH showed distant metastasis different from RH. We assume that RH could be a spectrum disease, possibly progressing and/or transforming into malignant angiosarcoma. Or skin specimen might be obtained only from a part showing the feature of RH that the primary mass could be composite HE, in which share features of at least two HE variants including angiosarcoma. Therefore, multiple biopsy is needed and workup of systemic involvement is necessary to evaluate a rare metastasis.

[P336] Generalized cutaneous angiosarcoma associated with multiple myeloma

( Jeong Won Jo ),( Young Bin Shin ),( Hae Bong Jeong ),( Yun Sun Moon ),( Do Seon Jeong ),( Eui Chang Jung ),( Chi Yeon Kim ),( Tae Jin Yoon ) 대한피부과학회 2017 대한피부과학회 학술발표대회집 Vol.69 No.1

Cutaneous angiosarcoma is a rare, malignant, vascular neoplasm of the skin commonly diagnosed in the head and neck regions. It arises infrequently in the lower extremity. In most cases, the exact etiology of cutaneous angiosarcoma is unclear. Recently, gene amplifications, especially MYC gene, have been identified as a new etiopathogenesis of angiosarcoma. MYC amplification may induce aberrant angiogenic phenotypes allowing the onset of the malignancy. Meanwhile, in multiple myeloma, MYC is activated and contributes to the malignant phenotype. Therefore, there is a possibility that MYC gene might be the common cause of angiosarcoma and multiple myeloma. Herein, we present the rare case of cutaneous angiosarcoma on lower extremity accompanied by multiple myeloma. An 85-year-old woman visited our department with multiple brownish to black colored nodules with hyperkeratotic crust, which were 0.3 to 0.8 cm in diameter, on the both feet and toes. The excisional biopsy was performed and a diagnosis of cutaneous angiosarcoma was made. At the time of visit, anemia, azotemia, proteinuria and hematuria were found. Immunofixation electrophoresis and bone marrow were performed, so multiple myeloma was diagnosed. We suspected carefully that MYC gene might be the common cause of angiosarcoma and multiple myeloma.

노인의 두피에 발생한 혈관육종 7예

박경덕 ( Kyung Duck Park ),정홍대 ( Hong Dae Jung ),이석종 ( Seok Jong Lee ),김병수 ( Byung Soo Kim ),이원주 ( Weon Ju Lee ),김도원 ( Do Won Kim ),서보익 ( Bo Ik Suh ),정호윤 ( Ho Yun Chung ),배한익 ( Han Ik Bae ) 대한피부과학회 2007 대한피부과학회지 Vol.45 No.12

Cutaneous angiosarcoma is a rare, aggressive vascular malignancy with a grave prognosis. It usually arises in the scalp or face as a locally-advanced patch, plaque or tumor at presentation and most often affects males and the elderly. Histopathologically, well-differentiated angiosarcomas are composed of well- or ill-formed vascular channels, often lined by flattened atypical endothelial cells and are distinguished from benign counterparts by their so-called collagen dissection pattern and anastomosing architecture. Varied differentiation may be observed even in the same tumor. Epithelioid angiosarcoma is a rare variant of poorly-differentiated angiosarcoma. The patients were seven cases of angiosarcomas including an epithelioid variant. They were six males and one female with an age range between 65∼84 years (avg. 74 years). Lesions resembled seborrheic dermatitis, erysipelas or spreading bruise that varied from blue to red in color and from papule to mass in size. They revealed satellite lesions far from the main lesion in some cases. Skin biopsies were performed for all patients and histopathologic features were compatible with angiosarcoma and epithelioid angiosarcoma. We herein report seven angiosarcomas of diverse clinical features because their early detection and precise differential diagnosis should be mandatory for effective management. (Korean J Dermatol 2007;45(12):1284∼1290)

A Case of Primary Bilateral Angiosarcoma of the Breast

이미자,김영숙,오형우,윤은주,이기욱 대한영상의학회 2016 대한영상의학회지 Vol.75 No.4

Angiosarcoma is a malignant tumor of endovascular origin that can occur in any part of the body including the breast; however, angiosarcoma of the breast is quite rare. There are two sub-types of breast angiosarcoma. One of the subtypes, primary breast angiosarcoma, is very rare; only a few cases have been reported to date. Bilateral primary breast angiosarcoma is even rarer, and several cases have been reported in postmenopausal women. We report a case of a 34-year-old woman with primary bilateral angiosarcoma. She had no other significant medical history, such as breast surgery or radiotherapy, which can lead to secondary angiosarcoma of the breast.

두피에 발생한 혈관육종의 치험례

최승석,유석근 대한성형외과학회 2002 Archives of Plastic Surgery Vol.29 No.6

Angiosarcoma is a rare malignant vascular tumor featuring endothelial cells. Angiosarcoma have four clinical manifestations that are angiosarcoma in the scalp and face, angiosarcoma in chronic lymphedematous areas, angiosarcoma in previously irradiated sites and malignant angioendothelioma. The most common lesions are those that occur in the scalp and in the face of elderly people. The male to female ratio is 2:1. In my case, a 65-years-old male presented progressively growing tumor on the left frontal scalp. The tumor began as an ill-defined bluish macule, which was initially mistaken for an old bruise. The rapidly growing lesion extended to form violaceousm compressible and hemorrhagic plaques. The mass was 6×4.5cm in size.Distinguishing features are the frequent occurrence of a peripheral erythematous ring, satellite nodules, the presence of intratumoral hemorrhage, and the tendency to bleed spontaneously, or after minimal trauma. Histologically the tumor comprised anaplastic pleomorphic endothelial cells with new vascular channels and irregular vascular spaces lined by plump endothelial cell's stroma among them. The lesion was diagnosed as angiosarcoma. No metastasis to other organs were found as evaluated by chest CT, bone and liver scan. We performed wide excision, skin graft, bilateral transposition flap, chemotherapy and radiation. The tumor was recurred at 4 months after surgical excision and with metastasis to the lung. The progress was characterized by an extremely rapid growth accompanied by distant metastasis. Surgery, irradiation and chemotherapy having no effect on the final outcome, the patient died 7 months after diagnosis.

P077 : A 13-year retrospective review of cutaneous angiosarcoma: Clinical characteristics and treatment outcomes

( Hee Su Kim ),( Ji Hee Kim ),( Hye Rang On ),( Mi Ryung Roh ),( Kyoung Ae Nam ),( Kee Yang Chung ) 대한피부과학회 2013 대한피부과학회 학술발표대회집 Vol.65 No.2

Background: Cutaneous angiosarcoma is a rare aggressive malignant vascular tumor with very poor prognosis. Because classic ‘Head-and-neck’ type angiosarcoma accounts for 50-60% of, particularly in elderly patients, diagnosis is late and treatment planning is often difficult. Objectives: The objective of this study was to retrospectively evaluate the treatment outcomes in patients with clinically localized angiosarcoma of the scalp and face. Methods: We analyzed the cases diagnosed as localized angiosarcoma between 2000 and 2012. The records of total 11 patients treated with radiation as a single modality or a combination of surgery, radiation therapy, and chemotherapy were reviewed. Results: 10 out of 11 patients were treated with radiation therapy and 4 out of 11 patients with both chemotherapy and radiotherapy. In only 2 patients complete resection was done due to the age and tumor burden. Patients treated with both radiotherapy and chemotherapy (2-year overall survival: 40%) had more favorable overall survival than patients treated with radiation single modality (2-year overall survival: 16.7%). Therefore, multimodal treatment with radiation therapy and chemotherapy was effective in improving overall survival for patients with angiosarcoma of the scalp and face. Conclusion: Although there was a limitation of the number of case, our data showed that radiotherapy with taxol chemotherapy may offer favorable prognosis for treatment of angiosarcoma in localized scalp and face.

혈관육종에 대한 임상 및 병리조직학적 분석

박동화 ( Dong Hwa Park ),김지연 ( Chi Yeon Kim ) 대한피부과학회 2015 대한피부과학회지 Vol.53 No.3

Background: Cutaneous angiosarcoma (AS) is a rare malignant tumor of endothelial origin with a very poor prognosis, frequent recurrences, and high metastatic potential. Clinical suspicion is often raised too late, but histological findings and immunohistochemical assays have proved to be very helpful in the diagnostic process. Objective: The purpose of this study was to evaluate the clinical and histopathologic features of angiosarcoma. Methods: We retrospectively analyzed 12 patients with angiosarcoma who had visited our department of dermatology in Gyeongsang National University Hospital between 1998 and 2013. The clinical features, evolution, treatment, and outcome were examined, and biopsy specimens were reviewed by a trained dermatologist, with subsequent immunohistochemical assessment. Results: Cutaneous AS was clinically diagnosed in 3 of 12 patients. There were many clinical misdiagnoses including lupus erythematosus, metastatic cancer, and infectious granuloma. The male to female ratio was 1:0.71 and the mean age of the patients was 66.25 years. The most frequent site of angiosarcoma was the scalp, in 8 of 12 patients. Histopathologically, well-differentiated angiosarcoma was composed of vascular channels, lined by flattened atypical endothelial cells that could be distinguished from their benign counterparts. Variable differentiation may be observed even in the same tumor. Immunohistopathologic analysis showed that the tumor cells were positive for CD31, CD34, Ki 67, and Factor VIII-related antigen. Conclusion: This study provides useful data on the clinical and histopathologic characteristics of angiosarcoma in the Korean population. (Korean J Dermatol 2015;53(3):188∼195)

눈꺼풀에 발생한 혈관육종 1예

유가영,정수경,백지선,양석우.Ga Young Yoo. MD. Su Kyung Jung. MD. Ji Sun Paik. MD. Suk Woo Yang. MD. PhD 대한안과학회 2013 대한안과학회지 Vol.54 No.7

Purpose: To report a case of angiosarcoma arising from the eyelid. Case summary: A 72-year-old male patient presented with swelling and erythema on the upper and lower eyelid of 10 months in duration. After the diagnosis of cutaneous angiosarcoma via tissue biopsy, no evidence of systemic metastasis was found. The right eyelid was treated with radiation therapy and a partial clinical response was achieved. After 4 months of follow-up, swelling and a red-purple plaque developed on the same (right) eyelid. Another biopsy was performed and the histological examination indicated a recurrence of angiosarcoma. Neither local nor distant metastases were observed. However, large-sized and ill-defined margins warranted size reduction by paclitaxel neoadjuvant chemotherapy, followed by surgical excision and eyelid reconstruction. Conclusions: Angiosarcoma commonly occurs on the face and scalp, but rarely occurs on the eyelids. Herein, the authors report a case of angiosarcoma arising from the eyelid.